ArticleJournal of inorganic biochemistry2018
Interactions of iron-bound frataxin with ISCU and ferredoxin on the cysteine desulfurase complex leading to Fe-S cluster assembly.
Article in Journal of inorganic biochemistry, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers.
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Who cites it
29 citing papers in PubMed, 57 citations in OpenAlex.
- Review
- Nanobodies as tools for studying human frataxin biology.Communications biology · 2026Article
- Iron-Sulfur Clusters and Iron Responsive Element Binding Proteins Mediate Iron Accumulation in Corneal Endothelial Cells in Fuchs Dystrophy.Investigative ophthalmology & visual science · 2025Article
- ATH434, a promising iron-targeting compound for treating iron regulation disorders.Metallomics : integrated biometal science · 2024Article
- Adeno-associated virus-mediated gene therapy in a patient with Canavan disease using dual routes of administration and immune modulation.Molecular therapy. Methods & clinical development · 2023Article
- Development of PPARγ Agonists for the Treatment of Neuroinflammatory and Neurodegenerative Diseases: Leriglitazone as a Promising Candidate.International journal of molecular sciences · 2023Review
- Protoporphyrin IX Binds to Iron(II)-Loaded and to Zinc-Loaded Human Frataxin.Life (Basel, Switzerland) · 2023Article
- Drosophila melanogaster frataxin: protein crystal and predicted solution structure with identification of the iron-binding regions.Acta crystallographica. Section D, Structural biology · 2023Article
- Characteristics of the Isu1 C-terminus in relation to [2Fe-2S] cluster assembly and ISCU Myopathy.Journal of biological inorganic chemistry : JBIC : a publication of the Society of Biological Inorganic Chemistry · 2022Article
- Skin fibroblast metabolomic profiling reveals that lipid dysfunction predicts the severity of Friedreich's ataxia.Journal of lipid research · 2022Article
- Structural and Functional Characterization of CreFH1, the Frataxin Homolog fromPlants (Basel, Switzerland) · 2022Article
- Investigation of bacterial diversity inBioengineered · 2021Article
- Iron-Sulfur Cluster Biogenesis as a Critical Target in Cancer.Antioxidants (Basel, Switzerland) · 2021Review
- Molecular Details of the Frataxin-Scaffold Interaction during Mitochondrial Fe-S Cluster Assembly.International journal of molecular sciences · 2021Review
- Enzymatic and Chemical In Vitro Reconstitution of Iron-Sulfur Cluster Proteins.Methods in molecular biology (Clifton, N.J.) · 2021Review
- Effects of FeInternational journal of molecular sciences · 2020Article
- A Highly Conserved Iron-Sulfur Cluster Assembly Machinery between Humans and AmoebaInternational journal of molecular sciences · 2020Article
- Hereditary Ataxia: A Focus on Heme Metabolism and Fe-S Cluster Biogenesis.International journal of molecular sciences · 2020Review
- Molecular Defects in Friedreich's Ataxia: Convergence of Oxidative Stress and Cytoskeletal Abnormalities.Frontiers in molecular biosciences · 2020Review
- Article
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
Abstract
Frataxin (FXN) is involved in mitochondrial iron‑sulfur (Fe-S) cluster biogenesis and serves to accelerate Fe-S cluster formation. FXN deficiency is associated with Friedreich ataxia, a neurodegenerative disease. We have used a combination of isothermal titration calorimetry and multinuclear NMR spectroscopy to investigate interactions among the components of the biological machine that carries out the assembly of iron‑sulfur clusters in human mitochondria. Our results show that FXN tightly binds a single Fe
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.